Histiocytosis

From Wikipedia, the free encyclopedia

Histiocytosis Classification and external resources
ICD-10	C96.1, D76.0
ICD-9	202.3, 277.89
eMedicine	ped/1997
MeSH	D015614

In medicine, histiocytosis is an excessive number of histiocytes,^[1] that is an excessive number of tissue macrophages, and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly it is sometimes used to refer to individual diseases.

According to the Histiocytosis Association of America, 1 in 200,000 children in the United States are born with histiocytosis each year.^[2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10. The University of California, San Francisco, states that the disease usually occurs from birth to age 15.^[3]

Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology.

Contents 1 Presentation 2 Classification, and relationships to other conditions 3 Common treatments 4 Organizations 5 References

[edit] Presentation

Histiocytosis is frequently associated with diabetes insipidus, even after several years of diagnosis and successful therapy.

[edit] Classification, and relationships to other conditions

There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the World Health Organization, they can be divided into three categories.^[4][5]. However, the classifications in ICD10 and MeSH are slightly different, as shown below:

Name	WHO	ICD10	MeSH
Langerhans cell histiocytosis (LCH)	I	D76.0	Langerhans-cell histiocytosis
Juvenile xanthogranuloma (JXG)	II	D76.3	non-Langerhans-cell histiocytosis
Hemophagocytic lymphohistiocytosis (HLH)	II	D76.1	non-Langerhans-cell histiocytosis
Niemann-Pick disease	-	E75.2	non-Langerhans-cell histiocytosis
Sea-blue histiocyte syndrome	-	-	non-Langerhans-cell histiocytosis
Acute monocytic leukemia	III	C93.0	malignant histiocytic disorders
Malignant histiocytosis	III	C96.1	malignant histiocytic disorders
Erdheim-Chester disease	-	C96.1	malignant histiocytic disorders

Types of LCH have also been known as "Eosinophilic Granuloma", "Hand-Schuller-Christian Disease", "Letterer-Siwe Disease", and "Histiocytosis X". (See LCH history for details).

[edit] Common treatments

• Chemotherapy
  • Cladribine (also known as 2CDA or Leustatin)
  • Etoposide
  • Vinblastine (Velban)

[edit] Organizations

Patients and families can gain some support by contacting patient organisations such as the Histiocytosis Association of America, which has several stable and proven treatment protocols available only for physicians,[1] or the Histiocytosis Research Trust.

[edit] References

1. ^ Stedman's Medical Dictionary. URL: http://www.emedicine.com/asp/dictionary.asp?keyword=Histiocytosis. Accessed on: May 24, 2007.
2. ^ Disease information at the Histiocytosis Association of America
3. ^ Histiocytosis - Signs and Symptoms
4. ^ Harris N, Jaffe E, Diebold J, Flandrin G, Muller-Hermelink H, Vardiman J, Lister T, Bloomfield C (1999). "The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997". Ann Oncol 10 (12): 1419–32. doi:10.1023/A:1008375931236. PMID 10643532. 
5. ^ eMedicine - Histiocytosis : Article by Cameron K Tebbi

v • d • e Pathology: hematology, hematologic disease (primarily D50-D77, 280-289) RBCs/anemia/ hemoglobinopathy (Myeloid) nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia) hereditary hemolytic anemia: enzyme disorders (G6PD Deficiency, Pyruvate kinase deficiency,Triosephosphate isomerase deficiency), Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia Polycythemia - Methemoglobinemia Coagulation/platelets (Myeloid) coagulopathy: DIC (Congenital afibrinogenemia, Purpura fulminans) • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease • Hypoprothrombinemia Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Factor V Leiden - Activated protein C resistance - Antiphospholipid syndrome other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Essential thrombocytosis - Thrombocytopenia Monocytes/Macrophages (Myeloid) WHO-I histiocytosis (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell histiocytosis (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant histiocytic disorders (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) -cytosis: Monocytosis -penia: Monocytopenia Granulocytes (Myeloid) -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome) Other/general myeloid Chronic granulomatous disease Lymphoid -cytosis: Lymphocytosis -penia: Lymphopenia Other Splenic disease (Asplenia/hyposplenism, Wandering spleen) - Pancytopenia See also hematological malignancy and immune disorders